Evans R A, Dunstan C R, Hills E
Calcif Tissue Int. 1983;35(1):5-8. doi: 10.1007/BF02404998.
A previously healthy 12-year-old boy developed pain on walking and x-rays showed osteoporosis. Over the next 2 years deterioration occurred, the condition became extremely severe, and he was confined to a wheelchair. After 5 years, marked kyphoscoliosis and pigeon chest deformity were present and little increase in height occurred. A wheelchair accident at the age of 17 resulted in several major long bone fractures. Iliac crest biopsies were taken at ages 15 and 17, and subjected to quantitative histology. A histochemical technique for osteoclast recognition by acid phosphatase activity showed resorption parameters to be normal. Double tetracycline labeling and histochemical identification of osteoblasts showed no abnormality of endosteal bone formation. Because of "coupling" of endosteal formation and resorption, these measurements might primarily reflect bone turnover. Failure of periosteal bone formation as shown by failure of radial growth of long bones and of epiphyseal growth was clearly evident. It is likely that osteoporosis developed in this patient due to a reduction in bone formation of unknown etiology rather than by increased bone resorption.
一名此前健康的12岁男孩出现行走疼痛，X线显示骨质疏松。在接下来的2年里病情恶化，状况变得极其严重，他只能依靠轮椅行动。5年后，出现明显的脊柱后凸侧弯和鸡胸畸形，身高几乎没有增长。17岁时的一次轮椅事故导致多处主要长骨骨折。分别在15岁和17岁时进行了髂嵴活检，并进行了定量组织学检查。通过酸性磷酸酶活性识别破骨细胞的组织化学技术显示吸收参数正常。双四环素标记和成骨细胞的组织化学鉴定显示骨内膜骨形成无异常。由于骨内膜形成和吸收的“耦联”，这些测量可能主要反映骨转换。长骨的径向生长和骨骺生长失败表明骨膜骨形成失败，这一点很明显。该患者骨质疏松很可能是由于病因不明的骨形成减少，而非骨吸收增加所致。
